An Autopsy Case of Mixed Connective Tissue Disease Complicated by Thrombotic Thrombocytopenic Purpura

Makiko Yashiro Furuya; Hiroshi Watanabe; Shuzo Sato; Yuya Fujita; Jumpei Tenmoku; Naoki Matsuoka; Tomoyuki Asano; Eiji Suzuki; Hiroko Kobayashi; Kiyoshi Migita

doi:10.2169/internalmedicine.3939-19

CASE REPORTS

An Autopsy Case of Mixed Connective Tissue Disease Complicated by Thrombotic Thrombocytopenic Purpura

Makiko Yashiro Furuya, Hiroshi Watanabe, Shuzo Sato, Yuya Fujita, Jumpei Tenmoku, Naoki Matsuoka, Tomoyuki Asano, Eiji Suzuki, Hiroko Kobayashi, Kiyoshi Migita

Author information

Makiko Yashiro Furuya
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan
Hiroshi Watanabe
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan
Shuzo Sato
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan
Yuya Fujita
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan
Jumpei Tenmoku
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan
Naoki Matsuoka
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan
Tomoyuki Asano
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan
Eiji Suzuki
Division of Rheumatology, Ohta-Nishinouchi Hospital, Japan
Hiroko Kobayashi
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan
Kiyoshi Migita
Department of Rheumatology, Fukushima Medical University School of Medicine, Japan

Keywords: mixed connective tissue disease (MCTD), thrombotic thrombocytopenic purpura (TTP), a disintegrin-like metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13), autopsy

JOURNAL OPEN ACCESS

2020 Volume 59 Issue 10 Pages 1315-1321

DOI https://doi.org/10.2169/internalmedicine.3939-19

Details

Abstract

We herein report a patient with mixed connective tissue disease (MCTD) who had been stable for years but suddenly developed thrombotic thrombocytopenic purpura (TTP). The patient showed a clinical pentad of signs of TTP, low activity of ADAMTS13, and positivity of anti-ADAMTS13 antibodies. She did not respond to plasma exchange or steroid therapy and died five days after admission. An autopsy revealed microthrombi in the brain, heart, kidney, adrenal glands, esophageal submucosa, and bone marrow as well as diffuse alveolar hemorrhaging. Physicians should bear in mind that TTP can occur in MCTD patients regardless of disease activity.

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